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  • Title: [Pulmonary arteriovenous fistula with Rendu-Osler-Weber disease and its HLA typing--a review and report of 87 cases of fistula].
    Author: Kawamura M, Takahashi Y, Sakata Y, Okubo T, Hoshino R, Kaneko K, Goko C.
    Journal: Nihon Kyobu Geka Gakkai Zasshi; 1994 Apr; 42(4):492-8. PubMed ID: 8035067.
    Abstract:
    Seven cases with pulmonary arteriovenous fistula (PAVF) in our hospital were reviewed on clinical data and HLA antigens. Furthermore, 80 reported cases of PAVF in Japan were summarized about the association with Rendu-Osler-Weber disease (Osler disease). Thirty-six (41%) of the total 87 cases had Osler disease. Fistulas were single in 46 cases and multiple in 41; unilateral in 56 and bilateral in 31. Operations were performed in 61 cases, embolizations in 8 and no therapy in 18. The frequency of the bilateral fistulas and multiple ones was significantly higher in cases with Osler disease (n = 36) than in those without Osler disease (n = 51) (p < 0.025). In our 7 cases, 6 had Osler disease. Aggregation of platelets was decreased in one case. Contralateral fistulas were recurred in another case 8 years after surgery. Three cases with Osler disease had the HLA halotype A26Bw6Bw61 which did not coincide with that in previous reports. We concluded that various HLA halotypes might exist in Osler disease.
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